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Has a dual function as a transcriptional activator and a repressor of the sonic hedgehog (Shh) pathway, and plays a role in limb development. The full-length GLI3 form (GLI3FL) after phosphorylation and nuclear translocation, acts as an activator (GLI3A) while GLI3R, its C-terminally truncated form, acts as a repressor. A proper balance between the GLI3 activator and the repressor GLI3R, rather than the repressor gradient itself or the activator/repressor ratio gradient, specifies limb digit number and identity. In concert with TRPS1, plays a role in regulating the size of the zone of distal chondrocytes, in restricting the zone of PTHLH expression in distal cells and in activating chondrocyte proliferation. Binds to the minimal GLI-consensus sequence 5'-GGGTGGTC-3'.
Gene Name: | GLI family zinc finger 3 |
Synonyms: | GLI3, ACLS, GCPS, GLI-Kruppel family member GLI3, GLI3 full length protein, GLI3-190, GLI family zinc finger 3, Gli-3, GLI3FL, PAP-A, PAPA1, PHS, PPDIV, Transcriptional activator GLI3, PAPA, PAPB, GLI3 form of 190 kDa, Oncogene GLI3, Zinc finger protein GLI3 |
Target Sequences: | NM_000168 NP_000159.3 P10071 |
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